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Referral and warning signs
When to refer
Refer to Haematologist/Specialist for: Diagnosis, Management of inherited disorders, Severe/recurrent bleeding, Need for specialized treatments (factors, IVIG), Anticoagulation reversal.
Red flags
- Spontaneous major bleeding
- Bleeding into joints/muscles
- Severe mucosal bleeding
- Associated sepsis/DIC
- Family history of severe bleeding
Signs and symptoms
- History of: Spontaneous bleeding (epistaxis, gums, GI, haematuria), Excessive bleeding after trauma/surgery/dental, Menorrhagia, Easy/spontaneous bruising (ecchymoses), Petechiae (pinpoint spots - platelet issue), Haemarthrosis (joint bleeding - factor deficiency), Deep muscle haematomas.
- Family history of bleeding disorders.
- Medication history (anticoagulants, antiplatelets, NSAIDs).
Risk factors
- Inherited disorders (Haemophilia, von Willebrand disease, platelet disorders).
- Acquired disorders: Liver disease, Kidney failure, Vitamin K deficiency, Malignancy (leukaemia), Autoimmune (ITP), DIC (sepsis, trauma), Medications (Warfarin, Heparin, DOACs, Aspirin, Clopidogrel, NSAIDs), Massive transfusion, Infections (dengue, VHF).
Diagnosis
Clinical assessment
History (type, severity, duration, triggers, family Hx, meds). Exam (petechiae, purpura, ecchymoses, haemarthroses, liver stigmata, splenomegaly).
Investigations
- Initial Screening: CBC (Platelet count), Peripheral Blood Smear (platelet morphology), Prothrombin Time (PT)/INR (extrinsic/common), Activated Partial Thromboplastin Time (aPTT) (intrinsic/common). Bleeding Time/PFA-100 (platelet function/vWD - less specific).
- Further Tests (Specialist): Specific Factor Assays (VIII, IX, vWF), Fibrinogen, D-dimer (DIC), Lupus Anticoagulant, Bone Marrow Exam.
Diagnostic criteria
Identify specific defect (platelet vs factor vs vessel) through clinical assessment and lab testing.
Management
Non-pharmacological management
- Identify and treat underlying cause.
- Stop offending medications.
- Local measures (pressure, packing).
- Avoid trauma.
- Genetic counseling.
Pharmacological treatment
- Depends on disorder:
- - Thrombocytopenia: Treat cause. Platelet transfusion if severe bleeding/very low count. Steroids/IVIG for ITP.
- - Haemophilia A/B: Factor VIII/IX concentrate. DDAVP (mild Haem A/vWD).
- - vWD: DDAVP, vWF concentrates.
- - Vit K Deficiency/Warfarin Reversal: Vitamin K. FFP or PCC.
- - Liver Disease: FFP, Vit K.
- - DIC: Treat cause, supportive (FFP, platelets, cryo).
- - Fibrinolysis: Tranexamic acid (specific situations).
Monitoring
Monitor clinical bleeding. Monitor relevant labs (Platelets, INR, aPTT, Factors).
Complications
- Severe/life-threatening haemorrhage
- Anaemia
- Joint damage (haemarthrosis)
- Treatment complications (transfusion reactions, inhibitors)
Prevention
- Avoid causative medications.
- Prophylactic factor (haemophilia).
- Genetic counseling.
- Safety measures.
Patient education
- Explain specific disorder.
- Avoid trigger meds (Aspirin/NSAIDs).
- Signs needing urgent attention.
- Activity restrictions.
- Prophylaxis need.
- Genetic implications.
- Medical alert ID.
Uganda-specific notes
Access to comprehensive coagulation tests and specific treatments (factors, IVIG, PCC) extremely limited. Diagnosis often relies on basic tests/clinical picture. Management focuses on treating cause, Vit K, FFP/platelets, local measures.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 11, Section 11.1.2